Our major goal is elucidating the pathophysiology of disorders of carbohydrate metabolism in infants and children. This has included studies of the basic metabolic pathways of glucose, cholesterol and fat in glycogen storage disease (GSD), utilizing studies in patients as well as in fibroblasts. CT technology was used to define glycogen and fat concentrations in liver both in animals and man. The studies of glycosylated hemoglobins in patients with GSD have been expanded. All of these studies have produced important leads and promising results. Studies of erythrocyte insulin receptors verified their increased numbers in patients with symptomatic hypoglycemia. These specific receptors have also been measured in patients with GSD and with hypopituitarism. Continuous glucose monitoring has begun to clarify the changes with controlled exercise stress in both children and adults as well as serving as a tool in monitoring induced hypoglycemia in patients with cancer. A wrist watch alarm which responds to a fall in temperature and sweating has proven useful in the early detection of symptomatic hypoglycemia in young infants and children.